Guillain-Barré syndrome in patients with Charcot-Marie-Tooth type 1A disease, probably a non-random association

IF 2.7 4区医学 Q2 CLINICAL NEUROLOGY

Neurophysiologie Clinique/Clinical Neurophysiology Pub Date : 2025-03-21 DOI:10.1016/j.neucli.2025.103071

Jean-Baptiste Davion , Jérôme Devaux , Céline Tard , Armelle Magot , François Cassim , Yann Péréon

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引用次数: 0

Abstract

Objective

To describe four cases of Charcot-Marie-Tooth disease type 1A (CMT1A) who developed Guillain-Barré syndrome (GBS), respectively the most frequent genetic and inflammatory neuropathies.

Methods

We described the patients’ clinical and electrodiagnostic characteristics.

Results

Our CMT1A patients developed typical GBS at various ages (3 to 76 years). GBS-related clinical manifestations were different within patients, with various severity degrees of seve (weakness, respiratory failure). Nerve conduction studies revealed more severe demyelinating features than expected in patients with no CMT1A. High cerebrospinal fluid protein level was found in 3 patients. GBS outcome was mainly good, although some patients only slowly improved.

Conclusions

Our cases are close to the previously described cases of acute worsening in CMT1A, and presented with many electrophysiological features of GBS. Overall, GBS prognosis does not seem worse in CMT1A patients than in other patients. If GBS and CMT1A were independent, the expected frequency of co-occurrence of GBS and CMT1A in our two French regions should be 1 case every 137 years. As we observed 4 cases in only 5 years, we suspect that CMT1A is a risk factor of GBS.

Significance

These cases bring further evidence for a non-random link between inflammatory and genetic neuropathies.

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来源期刊

Neurophysiologie Clinique/Clinical Neurophysiology 医学-临床神经学

CiteScore

5.20

自引率

3.30%

发文量

审稿时长

60 days

期刊介绍： Neurophysiologie Clinique / Clinical Neurophysiology (NCCN) is the official organ of the French Society of Clinical Neurophysiology (SNCLF). This journal is published 6 times a year, and is aimed at an international readership, with articles written in English. These can take the form of original research papers, comprehensive review articles, viewpoints, short communications, technical notes, editorials or letters to the Editor. The theme is the neurophysiological investigation of central or peripheral nervous system or muscle in healthy humans or patients. The journal focuses on key areas of clinical neurophysiology: electro- or magneto-encephalography, evoked potentials of all modalities, electroneuromyography, sleep, pain, posture, balance, motor control, autonomic nervous system, cognition, invasive and non-invasive neuromodulation, signal processing, bio-engineering, functional imaging.